53 yrs female with history/diagnosis of autoimmune throbocytopenic purpura.  
2013 threpine: reactive changes, probably induced peripheral hemato-cell consumption.  
2014 splenomegaly (laparoscopic) performed.  
MACRO: >50 spleen fragments from 0,5x0,5x0,3 cm up to 9,5x5x3,5 cm. Multiple brown elastic nodules up to iki 2,5x2x2 cm in the sections.  
HISTO: Multinodular infiltrating proliferation of highly atypical histiocytes with pleomorfphic, lobated nuclei and microvesiculated cytoplasm. Scattered megakariocytes with pleomorphic hyperchromatic nuclei and islands of erythroblasts with Ki67+ activity within infiltrate.  
IH: CD4+; CD68+;Lysozyme+; CD1a/S100(-); CD23/CD21(-). Ki67 prolif. activity low <5% (mainly higher on erythros, scattered histiocytic nuclei).  
OF NOTE: We have not more Ma markers there, I'm very sorry.  
 
Proposal: Histiocytic tumor/sarcoma with extramedullary haematopoiesis and hemophagocytosis.  
Question: Low Ki67 activity? Probablity of reactive changes?  
Dif. dx: Tumoral extranodal haemophagocytosis with prominent atypical histiocytic reaction. EXAMPLE OF THIS PATHOLOGY: http://www.ipath-network.com/ltpatho/object/view/372150  
 
Pubmed search:  
1. Primary splenic histiocytic sarcoma complicated with prolonged idiopathic thrombocytopenia and secondary bone marrow involvement: a unique surgical case presenting with splenomegaly but non-nodular lesions.  
Yamada S, Tasaki T, Satoh N et al. Diagn Pathol. 2012 Oct 17;7:143. doi: 10.1186/1746-1596-7-143. PMID: 23075171  
2. Oka K1, Nakamine H, Maeda K et al. Primary histiocytic sarcoma of the spleen associated with hemophagocytosis. Int J Hematol. 2008 May;87(4):405-9. doi: 10.1007/s12185-008-0059-6.  

tzankov 2014-04-29 07:30	very interesting case. the hemaophagocytosis is obvious. I would ask the lab if there were/are indeed proven auto-antibodies gainst platelets (and erythroctes) prior to further diagnostic suggestions. does the patient have any signs of a hemophagocytic syndrome?
ugnius 2014-04-29 19:41	Hematologists do not perform Ab tests there. Clinically any signs of hematophagocytosis present, but it was not suspected. Throbocyte counts restored after operation.     Additionally MACRO- I've seen a video of laparo-procedure- MULTINODULAR violet spleen.
tzankov 2014-04-30 08:27	since the lady does not have symptoms of hemaophagocytosis (ferritin? triglycerides?) I would prefer reactive histocytic proliferations rather than sarcoma (any atypia of the histiocytes, low proliferation fraction). If you want the study any phenotypic aberrations of the histiocytes you may add CD45, CD43, CD163, HLA-DR to test if the have "aberrant" phenotypes...
ugnius 2014-04-30 09:43	Agree on low Ki67, but caliber and nuclear atypia are still present. And macro view is impressive: I will try to catch video snapshot for demo. IH and B clonality pending.
ugnius 2014-04-30 12:08	Please find macro photo and video attached.
tzankov 2014-04-30 13:10	very impressive... but the atypia is very low grade if at all and the cells are functioning i.e. phagocyting. are there any phenotypic aberrations?
ugnius 2014-04-30 13:24	Yes..: I will proceed in day plus with IH except one absent still... (May Day 1: day off there in the best postsoviet traditions).
kunze 2014-05-04 12:08	Thanks, Dr. Mickys, for sharing this interesting case of a spleen with prominent nodular infiltrates of hemophagocytizing histiocytes. The moderate nuclear atypias and the low rate of MIB1-positive cells are in my view not well compatible with a splenic histiocytic sarcoma. I prefer a "nodular" hemophagocytic histiocytosis associated with an infection (particularly EBV) or an autoimmune disease. The splenic parenchyma between the nodular infiltrates shows a hyperplasia of the white pulp and scattered large "foamy" histiocytes in the red pulp as nonspecific findings which are frequently observed in AITs.
ugnius 2014-05-05 12:53	Thanx, but I have never met such catogory "tumor like LH"... Please find heterogenously positive HLA DR and LCA and negative CD43 IH.
tzankov 2014-05-05 13:10	indeed the histocytes have some phenotypic aberrations. now what?   considering the impressive morphology, the existing literture evidence and the subtle morphogic atypia and the pehnotypic abnormalities a careful consistency diagnosis with HS might be established. Yet, typically HS run aggressive courses and clinicians are tempted to give aggressive therpaies, which might not be proper here. Thus, all circumstances and cavetas need to be reported to the clinicains. Consider sendinb the case of John Chan in Hong Kong....
kunze 2014-05-07 14:21	I agree: the results of the immunostains for HLA DR and LCA tild the diagnostic balance towards a histiocytic neoplasia. But I share the objection to a conventional histiocytic sarcoma.
ugnius 2014-05-07 14:40	Thanx a lot. I've sent a consult request to dr. John K.C. Chan- we will proceed in case of success.
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